Huntington's Disease (HD) is a progressive neurodegenerative disease characterized by severe movement, cognitive, and behavioral changes caused by a CAG.CTG expansion in the Htt gene. Upon translation, this expansion mutation results in the production of huntingtin protein (HTT) with an expanded poly-Glutamine (polyGln, polyQ) repeat tract, which is toxic and contributes to disease pathology. While pre-symptomatic genetic testing for HD can identify the presence of disease-causing mutant Htt, a positive test does not necessarily indicate the initiation of the pathogenic process. Currently utilized HD therapies are limited to reduction of symptoms and do not provide a cure. Accordingly, novel compositions and methods for diagnosis and treatment of HD are needed.